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Organizing Pneumonia Associated With Common.46
Organizing Pneumonia Associated With Common.46
Organizing Pneumonia Associated With Common.46
Clinical practice
Organizing pneumonia associated with common variable
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immunodeficiency
CAO Meng-shu, CAI Hou-rong, ZHANG Ying-wei, MENG Fan-qing and SUN Ling-yun
YQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 04/09/2024
NK cells were normal. The histopathology of lung biopsy that in normal individuals. However, these B cells failed
samples obtained by VATS showed that plug of to mature into plasma cells in patients with CVID. The
granulation tissue obliterated the alveolar lumen, alveolar current patient had a lower peripheral blood B cell
ducts, and small airways (Figure 1C and 1D), and the number with normal levels of other immune cell subsets,
adjacent pulmonary parenchyma was infiltrated with which may result in lower serum levels of
chronic inflammatory cells. In local areas, there were also immunoglobulin. So far, the diagnosis of CVID remains
nodular lymphoid hyperplasia with germinal centers and exclusive, and the subjects must meet the criteria
fibrosis. Finally, she was diagnosed of OP associated proposed by Chapel and Cunningham-Rundles4 in 2009.
CVID and treated with oral prednisone 40 mg/d. She did Our patient was an 18-year-old girl, the serum levels of
not accept immunoglobulin replacement because of IgG, IgA and IgM were all below normal. The diagnosis
expensive medicine expenditures. of CVID was based upon a medical history of recurrent
respiratory tract infections associated with a dramatic
In the following month, pulmonary functions were greatly decrease in serum levels of immunoglobulins.
improved (Table 1). Chest CT (Figure 1E and 1F) showed
sparing strip opacities and GGOs at the middle and lower The common clinical manifestations of antibody
of the bilateral lungs, bronchiectasis of the right middle deficiency syndromes are repeated and prolonged
lobe, and mild reticular shadows at the bilateral basilar respiratory infections. However, the frequency and type
segments two months later. The extent of nodular of ILD in patients with CVID are incompletely
opacities and consolidations was markedly reduced. Then appreciated. Popa et al5 described that ILD was frequent
the dose of prednisone was tapered to 20 mg/d. Six (19.6%) in a consecutive series, much more prevalent
months after corticosteroid therapy, the white cell and than expected from general population surveys. Several
lymphocyte counts were normal, the percentage of B histologic patterns of ILDs have been reported in patients
lymphocytes in peripheral blood was still 1.2%. Serum with CVID, including lymphocytic interstitial
levels of IgG, IgA and IgM decreased to 0.1 g/L, 0.01 g/L pneumonitis (LIP),6,7 granulomatous interstitial
8
and 0.05 g/L, respectively. The patient refused pneumonitis, bronchiolitis obliterans organizing
immunoglobulins replacement treatment again. pneumonia (BOOP) or OP,2,3,9 and usual interstitial
pneumonitis.10 The most common pattern of ILDs in
CVID is a rare disorder characterized by the defection of patients with CVID is LIP, actually OP is quite rare. Up to
B lymphocytes function. Usually, the number of B cells now, only two cases of OP with CVID have been reported
in patients is lower or even not changed compared with in English2,3 and one case reported in Spanish.9 It noted
Chinese Medical Journal 2012;125(17):3195-3197 3197
that the patients with CVID who exhibit diffuse lung Acknowledgements: We thank Dr. LIANG Jun and Dr. WANG
disease were easily misdiagnosed as pulmonary infection Dan-dan for reviewing this manuscript.
as our patient.
REFERENCES
OP is a pathological feature of various diseases, such as
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infectious disease, autoimmune disorder, drugs, 1. Sneller MC, Strober W, Eisenstein E, Jaffe JS,
environmental and occupational factors. Infectious Cunningham-Rundles C. NIH conference. New insights into
disease was firstly eliminated in our patient through common variable immunodeficiency. Ann Intern Med 1993;
cultures and specific staining of the sputum and BALF. 118: 720-730.
Indeed, there was no evidence of collagen vascular 2. Wislez M, Sibony M, Naccache JM, Liote H, Carette MF,
YQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8KKGKV0Ymy+78= on 04/09/2024
disease because RF, ASO, ANA and ANCA were all Oksenhendler E, et al. Organizing pneumonia related to
negative; nor exposure to fumes, toxins or drugs known common variable immunodeficiency: case report and
to induce OP. After extensive evaluation of causative literature review. Respiration 2000; 67: 467-670.
factors, we speculated on the relationship between OP 3. Kaufman J, Komorowski R. Bronchiolitis obliterans
and CVID in view of two cases reported before.2,3 organizing pneumonia in common variable
Although our patient was absent of typical clinical immunodeficiency syndrome. Chest 1991; 100: 552-553.
symptoms of OP, her serial PFTs were consistent with a 4. Chapel H, Cunningham-Rundles C. Update in understanding
severe restrictive pattern and reduced gas diffusing common variable immunodeficiency disorders (CVIDs) and
capacity. Chest CT analysis showed migratory and patchy the management of patients with these conditions. Br J
consolidations and nodules of the bilateral lungs. Finally, Haematol 2009; 145: 709-727.
OP was definitively demonstrated by the pathological 5. Popa V, Colby TV, Reich SB. Pulmonary interstitial disease
findings. in Ig deficiency. Chest 2002; 122: 1594-1603.
6. Arish N, Eldor R, Fellig Y, Bogot N, Laxer U, Izhar U, et al.
There is no established guideline for the treatment of Lymphocytic interstitial pneumonia associated with common
ILDs in CVID. Most commonly, corticosteroids and variable immunodeficiency resolved with intravenous
immunoglobulins replacement monthly have been used to immunoglobulins. Thorax 2006; 61: 1096-1097.
treat these patients.1-3,6 There is no established guideline 7. Davies CW, Juniper MC, Gray W, Gleeson FV, Chapel HM,
on dosage and duration of treatment with corticosteroid. Davies RJ. Lymphoid interstitial pneumonitis associated with
Corticosteroids combined with immunoglobulin common variable hypogammaglobulinaemia treated with
replacement had been used in the two patients of OP with cyclosporin A. Thorax 2000; 55: 88-90.
CVID reported, which resulted in the improvement of 8. Delèvaux I, André M, Aumaître O. Wegener’s granulomatosis
clinical symptoms and radiographic abnormalities.2,3 Of associated with common variable immunodeficiency. J
note, Wislez et al2 reported the patient’s conditions Rheumatol 2002; 29: 1577-1578.
resolved after nearly 2 years of treatment. Interestingly, 9. Soto Campos JG, Cano Gomez S, Capote Gil F, Epler GR.
imaging abnormalities of the current case were markedly Bronchiolitis obliterans organizing pneumonia and common
improved only after oral prednisone without adding variable immunodeficiency. Arch Bronconeumol 1997; 33:
intravenous immunoglobulins. Thus, the standard therapy 548.
for OP in patients with CVID remains to be evaluated. 10. Walker JC, O’Connel MA, Pluss JL. Usual interstitial
pneumonitis in a patient with common variable
In summary, OP associated with CVID is very rare. To immunodeficiency. J Allergy Clin Immunol 1997; 99:
our knowledge, the clinicopathologic spectrum of ILD 847-851.
with CVID has not been reported in China. Surgical lung
biopsy should be considered in order to exclude
noninfectious diseases, especially in patients with CVID (Received February 16, 2012)
who exhibit diffuse lung disease. Edited by WANG Mou-yue and LIU Huan